Pseudotumor cerebri presenting as headache
Expert Rev. Neurotherapeeutics 8(3), 397–407 (2008)

Deborah I Friedman
University of Rochester School of Medicine and Dentistry, 601 Elmwood Avenue, Box 659, Rochester, NY 14642, USA

Tel.: +1 585 275 5332
Fax: +1 585 276 0292 deborah_friedman@

Pseudotumor cerebri (PTC) is characterized by intracranial hypertension without ventriculomegaly, in the absence of a mass lesion or meningeal process. When there is no secondary cause, it is termed ‘idiopathic intracranial hypertension’. Headache is the most common symptom of PTC, present in over 90% of patients. The headache is often disabling and nonspecific in character; thus, ophthalmoscopy is imperative for all patients being evaluated for headache. Visual loss is the major morbidity of PTC, requiring prompt diagnosis and treatment to prevent permanent deficits. Medical and surgical treatments are employed, although evidence-based treatment guidelines do not exist. This review discusses the diagnosis, differential diagnosis and management strategies for patients with PTC.
K EYWORDS: cerebrospinal fluid shunt • headache disorder • intracranial hypertension • obesity • papilledema
• pseudotumor cerebri • secondary

History: what’s in a name?

This disorder has been known by many names since it was first described by Taylor in 1880. Pseudotumor cerebri (PTC) was recognized as a distinct entity when Quincke published a detailed report in 1897 of ‘serous meningitis’ affecting several patients [1]. The term ‘pseudotumor cerebri’ was coined by Nönne in 1904, defining the clinical syndrome simulating a brain tumor in the absence of a tumor. In 1937, Davidoff and Dyke proposed the name ‘hypertensive meningeal hydrops’ after finding a history of otitis media in four of their 15 cases. Dandy published another 22 cases the same year of “intracranial pressure without brain tumor” and proposed that changes in intracranial volume of blood or cerebrospinal fluid (CSF) were responsible. Foley coined the term ‘benign intracranial hypertension’ in 1955 and realized that most of his 95 cases were idiopathic. Buchheit and colleagues debunked that term in 1969 noting that “the syndrome is neither a benign process nor a false tumor” as sev- eral of their patients lost vision. Unfortunately, Foley’s term continues to be used despite almost 40 years of experience supporting Buchheit. ‘Idiopathic intracranial hypertension’ (IIH) was introduced into the literature in the early 1990s and remains the preferred term when no secondary cause is identified. The original Dandy criteria for diagnosis were revised by
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Smith in 1985 to incorporate computed tomography, and further refined by Friedman and Jacobson to reflect modern imaging, asymptomatic patients and the occasional lack of papilledema [2]. Treatment in the mid-20th century consisted of subtemporal decompression or repeated lum- bar punctures. Weight reduction gained favor in the 1960s. Medical management with aceta- zolamide and furosemide, and surgical treat- ments of optic nerve sheath fenestration and shunting entered the scene in the 1970s. Many secondary causes have been described in the interim, but the mechanism of PTC remains uncertain. To date, there are no prospective treatment trials in PTC; thus evidence-based guidelines for therapy do not exist [3].


The idiopathic form of PTC generally occurs in patients under age 50 years, and most commonly affects obese women of childbearing age. The incidence of PTC in the USA has risen over the past 15 years, possibly attributable to the increas- ing prevalence of obesity or heightened awareness of the disorder. Current estimates place the inci- dence of PTC between two and 5.2 per 100,000 in the general population, thus affecting approxi- mately 20–22 per 100,000 obese women between the ages of 20 and 44 years [4,5]. Risk
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disturbed hearing. retinal infarcts. posterior or hemicranial cephalgia. This symptom is often not volunteered and must be specifically queried. and examined daily. if present.ated in the emergency department. Papilledema may be present early in the course. 398 . They produce an irregular optic-nerve contour that may simulate disc edema. if present. There is severe papilledema with disc hyperemia. including frontal. which is usually bilateral. with associated photophobia. severe periorbial pain and autonomic features rarely occur [9]. mass or abnormal enhancement. The location is variable. This may account for some cases of missed PTC in patients into a chronic daily headache [7]. It is uncertain whether it begins acutely or evolves over days to weeks. choroidal folds and macular edema may also be present (FIGURES 1 & 2). frontal and pressure-like [8]. torti. headache and diplopia for 2 weeks. Signs Headache is present in over 90% of patients and is usually the initial symptom. They do not correspond to the overall visual status. Optic nerve appearance (March 2007) in a 17-year-old female with blurred vision. loss of the physiologic cup and a peripapillary halo. A study of patients admitted to a neurosurgical intensive care unit with acute ICH of various etiologies. The visual obscurations usually last less than 60 secs and reflect papilledema. • Signs. are caused by intracranial hypertension (ICH) or papilledema.lar. Less com. • No other cause is identified (IIH). PTC is one of the few conditions causing transient visual obscurations. Cluster-like headaches with nocturnal awakening. as the headache of PTC is often similar to that of a primary headache disorder. Hemorrhages. nerve fiber layer hemorrhages. The headache is most frequently described as retro-ocu. The importance of performing ophthalmoscopy on all patients with new-onset headaches or a change in headache pat. vertigo.face of the optic nerves. moderate weight gain (5–15% of body weight) during the year prior to symptom onset. consisting of par. • Neuroimaging demonstrates no ventriculomegaly. They may be uniocular or binocular. • Normal mental status. Fever. found that only a small percentage developed papilledema by 1 week. Neck. meningismus or focal neurological deficits suggest another diagnosis. although they sometimes occur with optic disc drusen. some experts maintain that it develops before the patient becomes symptomatic.troversy is compounded by the technical difficulty of visualiz. are caused by ICH papilledema. Other visual symptoms include binocular diplopia. Low grade or early papilledema may be difficult to detect using a direct ophthalmoscope and an ophthalmological consultation is recommended. (2008) Figure 1. disc elevation. Symptoms Transient visual obscurations occur in approximately 75% of patients. throbbing headaches similar to migraine may occur. Stereoscopic viewing of the optic disc is also useful to distinguish true papilledema from pseudopapilledema Expert Rev.tern is underscored by these cases. blurred vision and subjective visual field loss. CSF rhinorrhea is a rare presenting symptom of PTC [10]. Disc drusen are calcifications that occur on or below the anterior sur. Pulsatile tinnitus is present in approximately 50% of PTC patients. She began taking doxycycline a few weeks the optic nerves well using a direct ophthalmoscope. the diagnosis is initially missed in many cases because of failure to examine the optic nerves.mon symptoms include ataxia. and the use of tetracycline antibiotics within 6 months of symptom onset [6]. The con. exudates. nor do they predict long-term visual outcome. However. often evolv. Diagnosis Criteria for diagnosis include the following [2]: • Symptoms.Review Friedman factors for developing PTC include female sex.collis and facial palsy. It may begin slowly or gradually. Neurotherapeutics 8(3). There is considerable controversy about the timing of papilledema in relation to the patient’s symptoms. or • Lumbar puncture shows an elevated opening pressure and normal CSF contents. A B The hallmark of PTC is papilledema. exudates and an early macular star.tial or complete visual loss that is often provoked by a change in posture. back or radicular pain is sometimes prominent. (A) Right eye and (B) left eye. and sometimes co-exist with disc edema. high body mass index (often over 30). but may be asymmetric or unilateral [11]. Neck. Features of papilledema that correlate with the severity of IIH include obscuration of the major vessels coursing over the disc margin. Her opening pressure was over 550 mm cerebrospinal fluid. The headache is frequently described as “the worst of my life”. However. phonophobia and nausea. back and shoulder pain are more prominent than headache in some patients.

mann) is required for the diagnosis and subsequent monitoring of the visual field. www.gence insufficiency’). may produce a existent. distention of the optic nerve sheaths.ous. both to exclude a secondary cause and to look for subtle changes suggestive of increased ICP. including discontinuities and filling defects from enlarged arachnoid granulations.logic blind spot.16]. are rare and suggest a secondary cause. which may be coparticularly superior sagittal sinus thrombosis. Magnetic resonance venography (MRV) frequently shows smooth-walled stenosis of the transverse sinuses [14]. as the stenosis resolves when the ICP is lowered [15. A unilateral or bilateral abducens nerve palsy may be present. Other venous sinus abnormalities may be present. Spontaneous venous pulsations usually suggest that the intracranial pressure (ICP) is below 250 mm CSF but their presence does not completely exclude increased ICP. Approximately 25% of the population lacks spontaneous venous pulsations. so their absence is not helpful as an early sign of papilledema unless one is certain that they were previously present. The exudate in the papillomacular bundle is more and gen. In most cases. Computed tomography may be performed urgently prior to the lumbar puncture but should be followed with MRI if possible. trochlear nerve palsy and generalized ophthalmoparesis. Venous sinus thrombosis. It is controversial whether MRV is necessary in every patient with PTC. A Chiari type 1 malformation is more common in patients with PTC than in the general population [13]. flattening of the posterior sclerae and protrusion of the optic nerve papillae into the vitre. Quantitative perimetry (automated threshold testing clinical syndrome identical to or Gold. Early visual (May 2007). Ventricular size is normal. this appears to be the result of the ICH rather than the cause of it. Other ocular motor deficits. Central vision is usually impaired late Figure 2. 5 weeks after bilateral optic nerve sheath in the course of papilledema but the fenestration. tiled optic A B nerves or optic disc drusen. Decreased visual acuity early in the course is an ominous prognostic sign and requires prompt treatment.Pseudotumor cerebri presenting as headache Review caused by anomalous discs. reflecting the natural pattern of resolution. MRI is recommended unless there is a medical contraindication or the patient’s weight exceeds the gantry limit. several multiple lumbar punctures and acetazolamide treatment visual field is involved early. (A) Right eye and (B) left eye. Indicators of increased ICP on MRI include an empty sella. this finding has generated much enthusiasm for employing venous sinus stenting to treat PTC. Nonetheless. Neuroimaging Neuroimaging is mandatory for patients with suspected PTC. Same patient as Figure 1. visual field (FIGURES 3 & 4) [12].future-drugs. Visual field constriction from IIH must be differentiated from non-organic visual field m 399 . inferonasal loss hyperemic with a peripapillary halo. producing binocular diplopia that is worse at a distance (‘diver. field defects include enlargement of the There is improvement in the papilledema although the optic nerves are still edematous and physio.eralized constriction of the distinct. such as oculomotor nerve palsy.

Increased ICP should be considered as a possible etiologic fac. noting that obese young women may also develop venous sinus thrombosis. signs and neuroimaging studies. but ICH is suspected with pressures greater than 100 mm of CSF for children under 8 years of age. Prolonged monitor. if treatment is initiated without the CSF examination.ting position. MRV should be performed in any atypical patient (e. Values between 200 and 250 mm CSF are indeterminate.toms. Cerebrospinal examination fluid A CSF examination measuring its opening pressure is required for diagnosis. www. the patient must be moved to the lateral decubitus position for an accurate is sometimes required as CSF pressure fluctuates. Normal values for CSF pressure are not well established in young chil. It is tempting to make a diagnosis based on symp.g..tor in obese patients with a relatively new onset of migraine without aura [17].racy of the diagnosis and need to continue therapy may be ques.PTC and is treated differently. Pretreatment of patients with anxiolytics may help in this regard. and over 200 mm of CSF for those between ages 8 to 12 years of age [18]. children or men) or in patients who do not improve with treatment. The diagnostic opening pressure should be 250 mm CSF or greater in adults. patients with normal resting opening pressures raised their pressure by 120–310 mm CSF simply by performing a Valsalva maneuver [19].future-drugs.tially extended. the accu. If the spinal needle is introduced with the patient in the sit. as the lumbar punctures may be technically difficult in an obese m 399 . Important secondary causes of ICH may be missed without a spinal fluid examination.tioned later without recourse. The patient must be relaxed with their legs par. or performed under fluoroscopy. Additionally. in one study. slim individuals. but foregoing the lumbar puncture is not advised.dren. Bilateral transverse sinus stenosis is occasionally found in patients with an otherwise typical migraine without aura.sure reading.

dient across the transverse sinuses in PTC nal and macular exudates may occur with severe papilledema that decreases acutely after CSF pressure is lowered [15. such as otitis gitis.16]. expanding uterus. intraocular pressure cerebral venous system influences CSF pressure. (B) Right eye: the blind spot is enlarged. Failure to measure the visual acuity in the times for normal homeostasis between the CSF and blood. It is imperative to perform quantitative pressure to increase. less clear. but improved since the initial examination. The best substantiated associated con(including cytology) are recommended on the diagnostic lum. CSF measurement. cerebral venous system and ICP. acute or chronic menin. visual field testing. a state characterized by Figure 4. Automated perimetry in the same patient as Figure 1 (March 2007). that produce ICH have no common mechanism or property that provides insight into the pathophysiology of the disVisual testing order. If so. any rise in central venous pressure causes CSF delay in treatment. increased venous blood volume and infeThe blind spots are still enlarged. The symptomatic response media with secondary venous sinus thrombosis. Automated perimetry in the same patient as Figure 1 (May 2007). one would expect a high incidence of PTC during pregnancy. The literature is ripe with case reports almost to fixation. (2008) . to CSF drainage is not diagnostic and postlumbar puncture The pathogenesis of PTC is uncertain. inflammation or malignancy. Manometry frequently demonstrates a phy is helpful in following the optic nerve appearance. of various conditions associated with ICH. (A) Left eye: there is marked enlargement of the blind spot which extends ers. Neurotherapeutics 8(3). A B Is it possible that elevated central venous pressure and decreased venous return from obesity leads to raised cerebral venous pressure and ICH? Some 30 30 investigators postulate that an inward intra-abdominal pressure gradient in central obesity is the primary mechanism for PTC in obese patients [22]. Recent reports focus on the relationship between the Ophthalmic assessment includes best corrected visual acuity. acute/emergent setting may contribute to misdiagnosis or a Therefore. Retipressure gra. The incidence of PTC 400 Expert Rev. There is no doubt that the pupil examination. High resolution MRV shows smooth walled Patients who experience permanent visual loss generally stenoses of the transverse sinuses in many PTC patients. some better substantiated than othFigure 3. rior vena cava compression from the (A) Left eye and (B) right eye. as the visual acuity and visual fields are the major may clinically mimic PTC [20]. The medications headaches may occur.21]. external examination and stereoscopic viewing pressure must be higher than cerebral venous pressure at all of the fundi. Macular exudates usually take several months to resolve and there may be a residual visual deficit after the fluid is reabsorbed. The relationship between venous sinus stenosis and PTC is factors guiding therapy. Prepubertal children are likely to bar puncture to exclude hemorrhage. although severe papilledema may well as fill. as have severe defects and irregularities in the contour be present with minimal visual sequelae.ditions are listed in BOX 1.Review Friedman A B 30 30 central acuity and it may be difficult to determine the relative contributions of macular edema and optic neuropathy to central visual loss. (FIGURES 1 & 2).have an underlying condition producing PTC. Macular involvement produces a decline in This suggests that venous hypertension and venous sinus stenosis is a result of the ICH rather than the cause of it [15. Etiology & pathogenesis There are many secondary causes of PTC. Fundus photograof cerebral veins [14]. This relationship accounts for the visual field testing by automated or kinetic (Goldmann) development of ICH with venous sinus thrombosis. which perimetry. making it hard to determine which The CSF protein is normal or low and routine studies factors are truly causal.

are not differ. All medications used to treat it are off-label. General measures • Levonogastrel (Norplant system) • Sulfa antibiotics guarantee relief of IIH symptoms. the pressure gradient is not inwardly directed unless the patient is in the supine position. Since many women with IIH have systemic fluid retention. There are no randomized trials of therapy in this disorder. The effect of weight loss on headache and the other associated symptoms of IIH have not been studied. retrospective data and experience. The postulated mechanism of vitamin A-related PTC is a toxic reaction between retinol and cellular membranes of the arachnoid gran. Although most patients with PTC have papilledema. it may be asymmetrical. Treating a secondary cause may not reverse the ICH rapidly enough to spare vision. The lack of papilledema in some eyes has been attributed to variable con. particularly If a secondary cause is identified. nor has any particular dietary protocol. Box 1. recent weight gain* • Orthostatic edema* • Polycystic ovarian syndrome • Addison’s disease • Hypoparathyroidism Exogenous agents • Chlordecone (kepone) • Corticosteroids (particularly withdrawal)* • Growth hormone • Leuprorelin acetate (leutinizing hormone-releasing hormone analogue) • Levothyroxine (children) • Lithium carbonate • Nalidixic acid Treatment ® Treatment decisions are generally based on visual status. Moreover. a low-salt diet that avoids excessive fluid intake may be helpful [28]. Weight loss is recommended for obese patients as a longterm management m 401 . Conditions associated with intracranial hypertension. Various hormones. This does not appear to be a result of obesity. Hypervitaminosis A is associated with PTC. Treatments used to restore/preserve vision may or may not improve headaches and vice versa. including estrogen. This corresponds to a concentration gradient of lipocalin-like prostaglandin Dsynthase between the spinal CSF space and the subarachnoid space surrounding the optic nerve [26].tinuity of the CSF subarachnoid space between the optic nerves and the brain. Impaired egress of CSF through the lymphatic system is another possible mechanism [25]. but CSF vasopressin levels are consistently higher in PTC patients than controls.future-drugs.ulations. The evidence supporting weight loss as an effective treatment is largely retrospective. although vitamin A is lipid soluble. A separate strategy is often required for headache management. Weight loss may improve the general health of morbidly obese patients but does not www. progestin. and some patients with IIH have high serum retinol levels and elevated CSF vitamin A levels [23]. unilateral or.cates compartmentalization of the intraorbital CSF space [26].Pseudotumor cerebri presenting as headache Review during pregnancy is the same as in control subjects. It is presumed that CSF has a bidirectional flow between the optic nerve sheath and the brain but computerized tomographic cisternography in patients with papilledema indi. the underlying condition is treated or the provoking agent is discontinued. Thus. absent. this mechanism seems unlikely. and suggests that losing approximately 6% of body weight is associated with an improvement in papilledema grade [27]. so the ICP should be treated concurrently as needed. impeding CSF absorption [24].ent in PTC patients compared to control subjects. and treatment strategies are based on case series. grehlin and leptin. uncommonly. Strict fluid restriction is not necessary. Obstruction to venous drainage* • Cerebral venous sinus thrombosis • Aseptic (hypercoagulable state) • Septic (middle ear or mastoid infection) • Bilateral radical neck dissection with jugular vein ligation • Superior vena cava syndrome • Increased right heart pressure • Cerebral arterial–venous sinus shunts Endocrine disorders • Obesity.

• Tetracycline and compounds* – Minocycline – Doxycycline • Vitamin A and compounds* related related • Accu tane – Vitamin m 401 .future-drugs. www. sustained weight loss. associations.nents of bariatric surgery for PTC postulate that its effective. The role of bariatric surgery is controversial. it pro. but this proposed mechanism has no physiologic basis.duces more rapid and. Propo. liver – All-trans-retinoic acid (for acute promyelocytic leukemia) Other medical conditions • Antiphospholipid syndrome antibody • Chronic obstructive pulmonary disease • Renal failure • Sleep apnea • Systemic erythematosis • Turner syndrome * lupus Common. well-established headache.ness is related to decreasing intra-abdominal pressure. generally.

37]. furosemide or other diuretics are used. Proponents of shunting over optic nerve sheath decompression surgery emphasize that it ‘treats the underlying problem’ of increased ICP. although they have never been prospectively evaluated. increased incidence or a lower threshold for surgical treatment in patients with PTC.tion since it may facilitate weight loss [29. Neurotherapeutics 8(3).lytes to avoid extreme acidosis or hypokalemia. the optic nerve may be protected from trabeculations of scar tissue between the optic nerve sheath and the optic nerve. may be used as tolerated. drowsiness and altered taste sensation (especially for carbonated beverages). Papilledema and visual loss must be present to consider this therapeutic option. is used as a first-line therapy to decrease CSF secretion from the choroid plexus. Once the fenestration heals. a carbonic anhydrase inhibitor. ocular discomfort and a tonic pupil. If carbonic anhydrase inhibitors are not successful.induced angleclosure glaucoma is an idiosyncratic reaction to the sulfa moiety.zolamide. corticosteroid use should be avoided in IIH patients. Higher doses. aplastic anemia and renal stones. In general. monitoring the electro.estration. Some patients may require both procedures if one surgical modality fails [35]. The surgeon places several fenestrations or excises a window in the optic nerve sheath. Surgery is not generally recommended for the treatment of headache alone. 65% of patients have improvement in their headaches and about half experience improved vision in the unoperated eye [36. and is probably caused by ischemia [40]. another carbonic anhydrase inhibitor. It may also cause mild anorexia and has a nonspecific therapeutic effect on headaches. tricyclic antidepressants. Stevens–Johnson syndrome. but shunts frequently fail.tions are generally minor and transient.triculoperitoneal shunts has oscillated over the years. which must be distinguished from precipitous visual loss of PTC. Aceta. Its weak carbonic anhydrase activity may be inadequate to decrease CSF produc. However. Consider using triamterene or spironolactone in patients who are allergic to carbonic anhydrase inhibitors and furosemide. Various types of shunts are employed to treat IIH. so its therapeutic benefit is for headaches of PTC rather than the visual status. This rise may reflect heightened awareness of the disorder. Optic nerve sheath fenestration is performed by an experienced orbital surgeon.mately 75% of eyes have improvement in the visual field. up to 4 g daily. verapamil. Mildly decreased serum bicarbonate levels are expected but do not generally require treatment. Care must be taken with co-administra. 402 Surgery is employed when patients have significant visual loss at presentation (based on visual acuity or visual field) or if their vision continues to deteriorate despite maximum medical treat. The mechanism underlying the effectiveness of the procedure is uncertain. counseling or pharmacologic treatment [34]. The decision to employ one or the other is often based on the available local the early dose-titration phase of treatment [31]. The preference for lumboperitoneal (LP) or ven. According to a national hospital admission database. some experts feel that corticosteroids may be detrimental in this setting. short duration of the procedure and amenability to outpatient surgery. Symptomatic treatment with migraine-specific medications may be are most commonly employed. Medications for headache prevention are useful.ble but requires prompt treatment by an ophthalmologist and drug discontinuation. the patient’s weight should be monitored carefully during treatment. As weight gain may exacerbate IIH. Papilledema usually improves following optic nerve sheath fenestration. the number of shunt procedures for IIH increased by 350% from 1998 to 2002. Shunting almost always works initially. such as topiramate. Aplastic anemia is idiosyncratic and cannot be predicted by routinely monitoring the blood count. Corticosteroids may be useful in the setting of acute visual loss to control the ICP prior to a surgical procedure. simultaneous procedures may be performed using either a medial or lateral orbital approach. The procedure is similarly effective in children [38] and patients with severe visual loss may improve or stabilize [39]. Methazolamide. Depression and anxiety are commonly present in patients with PTC and should be addressed with psychotherapy. Approxi. Topiramate is a preferred initial preventive medica.tion.tion of topiramate and acetazolamide. requiring long-term headache management [32].dure are the lack of hardware. Severe adverse reactions include allergy. Doses of 1–2 g daily are usually employed in adults. is an alternative to acetazolamide. as the ICP may rise as dose is tapered and because of weight gain [33]. (2008) . Visual loss resulting from the surgery occurs in less than 10% of patients. is generally binocular and usually occurs dur. Topiramate. including diplopia. Failure with recurrent visual loss may occur months to years later.Review Medications Friedman Surgical treatment Diuretics are routinely employed in the treatment of IIH. The major advantages of the proce. Unilateral (on the eye with the worst vision) or bilateral. with new shunt placements increasing by 320% [41]. There is an immediate lowering of CSF pressure in the subarachnoid space surrounding the optic nerve as fluid is released through the fen. but the use of daily analgesics should be avoided to prevent medication-overuse headache. Common side effects of acetazolamide include paresthesias. It is reversi. Headaches from IIH respond variably to diuretics but are managed medically.30]. Neither procedure has been studied prospectively nor are there studies directly comparing the two procedures. compartmentalization of the optic nerve or from a filtering mechanism. Patients with IIH commonly continue to experience headaches after their ICP is successfully treated. radiculopathy and a high Expert Rev. Optic nerve sheath fenestration and shunt. PTC is commonly treated using LP shunts. gabapentin and valproate. The complica. Topiramate rarely produces acute angle-closure glaucoma. Their disadvantages include overdrainage with an acquired Chiari malformation or low-pressure headaches.

(2008) . In a retrospective review of 27 IIH 402 Expert Rev.incidence of shunt failure. Neurotherapeutics 8(3).

In this series. but 75% failed by 24 months postinsertion. Visual loss may be the first evidence of shunt failure [45]. Two patients had severe visual loss and eight had chronic papilledema. At follow-up. 19% underwent three to five and 12% had six or more procedures. The discovery of venous sinus stenosis in PTC patients raises the possibility of using venous sinus stenting to treat it. 56% required revision. The initial report of stenting for PTC included 12 patients with intractable headaches and a visual disturbance lasting 5 months to 12 years [46]. intracranial noises and transient obscurations of vision).Pseudotumor cerebri presenting as headache Review patients treated with LP shunts with a mean follow-up of 6 years. two had improved except for residual headache and five were www. Shunt failure may be difficult to verify. and they were twice as likely to become obstructed compared to ventricular shunts. This presumes that increased pressure in the dural venous sinuses is the cause of increased ICP. A total of 45% of patients had one procedure. Other manifestations of shunt malfunction are the return of headaches. Interventional radiological treatment unchanged. Ten patients underwent unilateral sinus stenting only and two underwent contralateral stent placement CSF diversion procedures in 37 patients (73 LP and nine ventricular shunts) showed an average time to shunt failure of 9 months (range from 1 day to more than 15 years) [43]. or the development of other PTC symptoms (e. All had previously received medical treatment. five patients were asymptomatic.future-drugs. optic nerve sheath fenestration or a shunt procedure. lack of papilledema and symptoms lasting longer than 2 years were risk factors for treatment failure.triculoperitoneal shunts have the advantage of incorporating programmable valves. The most common reasons for shunt revision were shunt failure and low CSF pressure [42]. a concept that is controversial with much evidence to the contrary. Ven.4 per patient with a median time to first revision of 11 months. The procedure may help in some cases and has inconsistent effectivness. as radiographic continuity does not ensure patency. Ventricular shunts inserted with frameless stereotactic guidance were all successfully placed. The average revision rate was 2. LP shunts were more likely to require revision (86%) than ventricular shunts (44%).co m 403 . No surgery had been performed within 10 months of stenting. Another study review. although their utility in PTC has not been confirmed. Sometimes the site of dysfunction is only found at the time of shunt revision. headaches recurred in 48% of patients by 36 months. visual function was not assessed. Review of a 30-year experience with shunting for PTC at Johns Hopkins School of Medicine studied 115 shunt procedures (79 LP and 36 ventriculoperitoneal or ventriculoatrial) in 42 patients [44]. 24% underwent two..g. Although 95% had a significant improvement in headaches 1 month after the procedure. marked worsening of headaches. LP shunts were more prone to overdrainage and tonsillar herniation.

If necessary.age. Ch ild re www. Five patients had partial transverse sinus obstruction and four were treated with stents.nant women are successfully managed using acetazolamide and intermittent lumbar m 403 . is not currently recommended for routine use. Corticosteroids may also be used. and re-stenosis of the venous sinuses may occur. Venous sinus thrombosis should be considered when PTC develops in the peripartum period. transient hear.Papilledema resolved in four patients and improved in one. Venography after stenting showed a reduction in intrasinus pressure that did not correlate with clinical improvement. One patient with complex venous anatomy was not stented but had a shunt placed with a successful outcome. five of whom had numerous revisions) and subtemporal decom. Most preg. All had papilledema prior to stenting that resolved after the procedure. Complications of stenting include headache. Six of seven had long-term improvement in vision that was dramatic in one case (light perception to almost normal vision within weeks). surgery may be performed.pression (one patient). Medical treatment options for headache are limited and sodium valproate is contraindicated. and obesity is present less often than in adults [49]. whilst another had resolution of bilateral abducens palsies. still in its exploratory phases. Stenting. Four patients were feeling well and required no additional treatment. preg. The duration of disease and previous treatment were not described in the report. optic nerve sheath fenestration (three patients). Among adolescents.nancy is not contraindicated in women with PTC. transient unsteadiness and one lifethreatening acute subdural hematoma [47]. The authors concluded that IIH may be caused by dural venous sinus thrombosis in some patients and cause venous stenosis in others. Seven patients had headaches over the stenting site that resolved in days to weeks. n Diagnostic criteria for PTC in children do not currently exist and are proposed by Rangwalla and Liu (BOX 2). girls are affected Special circumstances Preg nanc y Although PTC may develop or recur during pregnancy.ous modalities including acetazolamide (three patients). Prior to puberty. Nine consecutive patients at another center were evaluated with direct retrograde cerebral venography and manometry. The subdural hematoma developed during venography and stenting in one patient who also had an optic nerve sheath fenestration and external ventricular drain. PTC affects boys and girls equally. with simultaneous CSF pressure measurement in two patients [48]. An additional eight patients with IIH were subsequently stented [47]. All patients had been previously treated with vari. optic nerve sheath may be preferred to surgically treat visual loss. Since the expanding uterus may obstruct the shunt catheter. The procedure is painful if general anesthesia is not employed. shunt (six patients.

Approximately half of patients improved with lumbar punctures or dihydroertgotamine. • No evidence of hydrocephalus. • Cranial nerve palsies allowed if they are of no other identifiable etiology and improve with reduction in cerebrospinal fluid pressure or resolution of other signs and symptoms of intracranial hypertension. • If symptoms or signs present. IIH with normal CSF pressure by lumbar puncture has also been described.* [18]. apathy. • Documented elevated intracranial pressure (age appropriate) measured in the lateral decubitus position. IIH variants A case–control study described IIH without papilledema (n = 25) in overweight women seeking care for chronic daily headache at a headache center [54]. Papilledema is occasion. growth of pubic hair or menarche. they may only reflect those of generalized intracranial hypertension or papilledema (normal mental status). supported by no evidence of pubic hair. These patients require aggressive management. the visual outcome may be poor despite rapid intervention [53]. Prognosis The disorder is usually monophasic and self-limited.ache being the most pervasive residual symptom. – Age less than 8 years with papilledema: less than 100 mm H20 – Age 8 years or above or less than 8 years without papilledema: less than 250 mm H20 • Normal cerebrospinal fluid composition except in neonates who may have up to 32 white blood cells/mm3 and protein as high as 150 mg/dl. discovered in asymptomatic children during a routine eye exam [52]. some patients continue to have lifelong symptoms. If the skull sutures are still open. After excluding a secondary cause. structural or vascular lesion on MRI. a stiff neck. However. headaches improved but most patients required more than one procedure. employing medical and surgical measures. There may be marked papilledema. mass. Fulminant (‘malignant’) IIH with head. A third of patients responded to diuretics. these children may be followed conservatively if their visual function is normal. Such patients generally have marked visual field loss or decreased central visual acuity at presentation.use headache in both groups of subjects. papilledema may be mimimal or absent. Shunting was performed in five patients. ataxia. Management of IIH in children is similar to adults. more frequently than boys. 404 Expert Rev. Proposed diagnostic criteria for idiopathic intracranial hypertension in pre-pubertal children. Neurotherapeutics 8(3). • No other identified cause of intracranial hypertension. A secondary cause is frequently found. Narrowing of the tranverse sinuses is allowed. *In boys. Reproduced with permission from [18]. evidence of optic nerve ischemia and macular edema. In girls. irritability or tor.Review Friedman Box 2. medication or otitis media [51]. IIH in young children may present with somnolence. There was a high rate of medicationover. Compared with control subjects (n = 60).ticollis [50]. such as infection. supported by lack of breast development. those with ICH were more commonly obese and had pulsatile tinnitus. Episodic or In some cases. the manifestations of IIH appear rapidly with early visual loss. with and without contrast. (2008) . and MR venography.

anemia. Neurotherapeutics 8(3). atrophic papilledema. although residual visual field defects persist in up to 50% of patients when assessed by sensitive perimetric methods. Overall quality of life is impacted upon in IIH. Patients with IIH have significantly lower health-related quality of life and visionspecific health-related quality of life (assessed by SF-26 and NEI VFQ-25. There are little prospective data assessing the long-term visual prognosis in PTC.ual loss are high grade papilledema. optociliary shunt vessels. This is particu. sub. or migraine without aura is present in over two thirds of patients after other symptoms and signs of ICH resolve. Depression and anxiety are common. Factors associated with vis.larly problematic in patients with residual optic atrophy. and not attributable to obesity. Failure to diagnose PTC in the acute stages often leads to a 404 Expert Rev. The most common persistent visual field defects are generalized vis. It may be difficult to discern whether the persist. intraocular hypertension. Visual acuity loss at presentation and rapid decline of visual function early in the course are unfavorable prognostic indicators. respectively) scores compared to neuro. The visual outcome is generally good. (2008) . as their optic nerves may not swell in response to high ICP. arcuate scotomas and enlarged blind spots.ophthalmologic control patients and a disease-free reference group.retinal hemorrhages at presentation.chronic tension-type headache. systemic hypertension and renal failure.ual constriction. and often requires long-term treatment [32]. Recognition of the disorder seems to be improving but the diagnosis is often initially missed. Expert commentary The incidence of PTC is rising in parallel with the increase in obesity in the Western world. particularly in the emergency room/urgent care setting. Legal blindness or complete blindness occurs in less than 5% of patients.ence or return of headaches in patients with IIH represents recurrent disease or a benign headache disorder. superior or inferior nasal field loss.

preferably by physicians familiar with the treatment of this disorder. Weight loss seems to be help. ophthalmologists. If successful. present in over 95% of patients at diagnosis. In general. such as auto-triggered. The management of PTC requires a team approach. “There is insufficient information to generate an evi. elliptic-centric-ordered (ATECO) 3D-enhanced MRI will continue to enhance our ability to diagnose PTC and detect cerebral venous abnormalities.dure is more effective than the other. systemic hypertension and renal failure.tive PTC studies have produced ambiguity regarding thera. Management is best guided by a neurologist or neuro-ophthalmologist. Recurrence is possible and headaches often persist after the intracranial pressure is controlled. and the lack of funded research on PTC to date is disappointing. Visual prognosis. commensurate with the rise in obesity in the Western world.ments available. anemia. Patients with chronic daily headaches and no papilledema may also have ICH.tus of ICP. • Pseudotumor cerebri (PTC) is characterized by intracranial hypertension without ventriculomegaly or a mass lesion. Non-invasive alternatives for measuring ICP are being studied. neurologists are in the best position to take charge of PTC management. optic nerve examination by fundoscopy is important when evaluating headache patients. idiopathic intracranial hypertension (IIH). Obesity is a recognized risk factor for chronic daily headache. although there are currently no evidence-based guidelines for treating PTC. most frequently affects individuals under age 45 years. www. and individuals of normal weight.Pseudotumor cerebri presenting as headache Review delay in treatment and increases the risk of permanent visual loss. although it will probably not replace the diagnostic lumbar puncture in most cases. A team approach is required for optimal care. A neuro-ophthalmologist should be involved whenever possible. Key issues Headaches are managed medically. evidenced-based guidelines for treating this group of patients will be available in approxi.peutic strategies. Five-year view Advances in neuroimaging.dence-based management strategy for IIH. Of the various treat. but the ongoing studies of stenting will determine its role in the treatment armamentarium.future-drugs. • Both medical and surgical treatment options exist. and exclusion of secondary causes.tration in patients with moderate visual loss.ful in the long term in obese patients. • Headache is the most common symptom of PTC. The idiopathic form. Properly designed and executed trials are needed” [3]. Currently. Transient visual obscurations and pulsatile tinnitus are common initial symptoms. but require input from the ophthalmologist regarding the patient’s visual status. especially men. Venous sinus thrombosis may simulate IIH and should be considered in all patients. including critical input from an ophthalmologist regarding the patient’s visual status. pregnant or post-partum women. There are no specific characteristics of the headache that differentiate the headaches of PTC from other conditions. although a small percentage of patients go m 405 . NORDIC submitted two treatment trial proposals to the NIH in 2006. • Risk factors for permanent visual loss include severe papilledema. Thus. there is inadequate information regarding which are truly beneficial and which are potentially harmful. visual loss at onset and early deterioration of vision (with or without treatment). a lumbar puncture with opening pressure measurement and spinal fluid examination. Should the study of mild visual loss commence in 2009. Neurologists. • The incidence of PTC is increasing. patients with normal vision or mild visual loss are treated medically. it is uncertain whether or not chronically elevated ICP is a contributing factor or a confounder [55]. neurosurgeons.mately 5 years. In the words of Lueck and McIlwaine from their Cochrane review. this option will be helpful for screening patients with suspected ICH and monitoring the sta. Secondary causes are treated concurrently with management of the ICP. • The prognosis is generally good. Medical management is preferred for headache treatment. although they are often described as the worst headache of one’s life. Thus. • Diagnosis requires neuroimaging. • The goal of treatment is to preserve vision. it is important to perform fundoscopy in all patients evaluated for headache to assess for papilledema. • Prompt diagnosis and treatment are needed to prevent permanent visual loss. particularly obese women of childbearing age. These patients may have undetected obstructive sleep apnea provoking their headaches and ICH. although treating the ICP is generally unsatisfactory. and those with moderate or severe visual loss (based on visual acuity and visual field) are candidates for surgery. including one study of medical management for patients with mild visual loss and a second study incorporating early optic nerve sheath fenes.tion to shunting was explored by the Neuro-Ophthalmology Research Disease Investigator Consortium (NORDIC) but deemed unfeasible owing to sample size constraints and the lack of convincing evidence in the existing literature that one proce. A study comparing optic nerve sheath fenestra. It is unlikely that additional new treatments will emerge within the next 5 years. radiologists and primary physicians are involved in the care of these patients and close communication amongst all providers is paramount. headache disability and the effect on quality of life will be established using these prospective data. The lack of evidence-based treatment guidelines and prospec. The disease is not rare.

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